*intro Music* 2000 families in Britain have a child born with a genetic disorder that makes them fixated by food. She thinks about food almost twenty-four seven. I wouldn’t be surprised if she actually dreams about it sometimes. There’s one occasion where she ate cat poo *inaudible* Rebecca’s life revolves around food. That’s her whole life. *inaudible* And it does cause a lot of tension. The urge to feed the hunger can drive people to desperate lengths When I was younger I used to rummage bins and when I got angry I used to kick the kitchen door down The whole front bit came out as you can see. It’s cracked in here What are you on about!? He has attacked me in the past You don’t know when hes going to flip so it’s like living with a wild animal If I eat more and more I will just… die Weight gain can be rapid. Leading to obesity, disease and early death It’s like an obsession that takes her over. I eat chocolates and biscuits and stuff She is not capable of controlling her own weight I said it was like a prison cell and it is like a prison cell If she had complete freedom… she would eat herself to death *Crying* Wake up, its time to get out of bed darling. You alright? I knew something was wrong when I was pregnant She shouldve been… moving inside of me which she wasn’t It was just the worst case scenario Rebecca Medley is eleven years old and already weighs eleven stone (154 lbs) She was born with a genetic disorder called Prader–Willi syndrome or PWS They’re classed as mentally retarded. They’ll get to big sizes and die very early because of heart problems. PWS is caused by deletion or damage to chromosome 15. The effects are physical, mental, emotional and incurable. I said, we might as well just kill her now then. There’s no point in her going on Children with PWS soon develop a powerful obsession with food The eating must have come in when she was about seven or eight. She used to steal fruit from the fruit bowl, other people’s food off their plates if they weren’t watching. Uh, she would go down the bins Rebecca would eat almost anything I remember the day that she ate the cat poo and I had to wash her mouth out. I mean it was nothing to her.. But the taste must’ve been vile Some families resort to locking up food cupboards Rebecca’s house has an alarm in the kitchen. So, if Rebecca comes this far the alarm will pick her up and the alarm will go off Despite all this, Rebecca is getting bigger and her problems look set to multiply They don’t make school uniforms in Rebecca’s size. Do they? So one of Anna’s relatives has made us some clothes Are you gonna go down and do your lunch box and Daddy will check it in a minute? Rebecca’s brain never signals when shes full up. So she’s always hungry Rebecca thinks about food, I think, every minute of our life. And it’s food that’s often the cause of her temper tantrums Mummy, they’re not Chloe’s! You have to share them, Bec. When Rebecca goes into one of her tantrums it can be absolutely over nothing or it could be over something really important. Rebecca’s lunch box is checked every day to make sure she’s not sneaked in something naughty But this morning she’s more interested in the cat food What are you doing with that cat food? Just looking at it. I’ve got to sort all this out – What flavour is it? I don’t know. I mean, I’m not about to try it to find out, Becksy. I eat cat food. Do you? I know you’ve tried the dry food. A bit crunchy, innit? When’s that then? Before I go to bed. I don’t believe you. What does it taste like? Meat. It’s not just food – PWS causes a wide range of learning disabilities So Rebecca gets special needs support at her local primary school. I do worry sometimes about when Rebecca goes to school about what sort of a day she’s gonna have Whether she’s gonna be happy How many boys are gonna pick on her today Prader–Willi syndrome is caused by a damaged chromosome, but it affects the whole of Rebecca’s body. It’s not just the case of the eating, which is the main issue, where the brain doesn’t tell the stomach it’s full up. It’s her sexual development, it’s her mental development, it’s her social development. It’s everything About 2000 people in Britain have PWS. There is no cure but, with careful management can it be controlled? At 18 stone (252 lbs) Katie Williams has fought the disorder for all of her 23 years When she was born we were told she’d be dead by the time she was in her late teens or early twenties She would’ve over eaten herself to death She was slim… till she left school and then college was bad and then since she’s finished college it’s disastrous It’s a cruel twist of PWS that Katie needs only half of the calories needed by the average women. This is because her poor muscle tone doesn’t burn up calories as fast Loads of things under 300 calories. Katie’s daily limit is just 900 calories She loves biscuits she’s never allowed to have them. She never has chips, or burgers, or fried things, or roast potatoes… It’s quite difficult when you think of how they’re all pleasures for the rest of us. Katie lives above the pub run by her divorced dad. She is relatively independent – much more so than many people with Prader-Willi Syndrome. But her freedom comes at a price: Not only does dad count her calories. He also counts her cash. Generally speaking if she wants something, I give her money for it. But I like to see receipts when she comes back Got some change for me? Yes. Now I partly live independently, cooking my own meals and I choose what I want to eat Katie keeps hunger pangs at bay with low calorie lunches and heaps of vegetables for her evening meal Katie believes she can live independently with PWS Will Katie ever realise her dream? And just why, despite her dad’s best efforts, is she piling on the pounds? She won’t admit this: if she had complete freedom, she would eat herself to death Its’ not just her size that makes life difficult for 11 year old Rebecca Medley. PWS gives her weak muscles and a lack of coordination. This makes even the simplest physical activity like getting up off the floor an ordeal Like a big beached whale is my Becky. She can’t just get up like you or I would do. She would have to roll over and go on her knees probably, and climb up something. She couldn’t do it quickly Suppose if she was sat at dinner she might get up a bit quicker, But no, she does everything in slow motion. Rebecca is never out of hospital with her weight and growth-related problems. I think where her spine has begone to curve she has been getting a lot of pain so I think maybe she’s looking into where there was a problem with her spine Put this ear here. Put this ear here Does that hurt? No Does that hurt? no Does that hurt? yeah. It does? yeah. I’ve seen enough. When people look at Rebecca now they just assume she’s just a very overweight eleven year old They wouldn’t say “That child’s got PWS” Even in hospital, staff can misjudge the impact of a treat. Lady: I might be able to find you a sweeties. Would you like a couple? Mom: No, just the one!
Lady: Oh, Just the one? Lady: It is nearly Christmas mom
Mom: No, just the one! Lady: Would you be aloud to take a couple?
Mom: No, thank you, Just the one.
Lady: Oh, dear. If you’re given more than one at a time next time she goes back she’d want one or more. So, I think one was enough. Wasn’t it? It’s 6:30 in the morning in Bristol. Time for 23-year old Katie Williams to get up for work She wears an oxygen mask because her weight is beginning to cause her breathing difficulties Katie’s breakfast just consists of a small sachet of flavoured porridge, made with water. That’s 125 calories, but before she can eat it, she is weighed by her dad. Katie has put on three stone in ten months. That’s 116.8 She now weighs over 18 stone. (252 lbs) She’s clearly overweight, and I do weigh her every day. So I can tell within a day or two, whether she’s been cheating or not. I use the word “cheating”. She uses the word “nibbling”. Because that’s a better self-image, I think. Tony gives Katie £1 a day. It’s meant to buy a cup of tea on her way to work. Can she be trusted? This is a shop I normally go to on the way to and from work. The danger for Katie is that just one packet of biscuits can easily exceed her daily limit of 900 calories. I’d buy a box of those, and eat the whole box. Katie resists the temptation. But it’s only minutes since breakfast and she’s already feeling the hunger. It’s that sort of feeling for hunger. Katie travels to work prepared. I normally bring an apple with me or some veg, and I eat that. She couldn’t survive the two bus journeys to work without her precious tea money. Every day is a struggle, but Katie believes she can beat PWS with her own willpower. Many people like us are in homes with the cupboards locked and no freedom. I think people should have the opportunity to learn to cope with not nibbling food, or anything like that. But can Katie herself cope without nibbling? Working helps. Katie is one of the few people with PWS to have a full-time job. She is a cleaner in a special needs school. The only problems I have could be walking up stairs. You get out of breath when you get up to the top. Lot of stairs! If you do a lot of work, like cleaning the rooms and stuff, I would have to sit down. My legs get achy. It’s hard to resist the lure of the staff canteen. Temptations here is biscuits and toast. Katie can’t resist; her low calorie lunch is supplemented by a wafer biscuit. It’s cheating like this that makes her weight creep up, and alerts her dad to take action. Well, I don’t search that often. It’s only when her weight’s going up, and she doesn’t like it. Fruit Pastilles. At least it’s not chocolate. But that’s chocolate. That’s a packet of Rolos, so that’s not good. I don’t think Katie’s ever going to be able to control her weight on her own without help. If she had access to money, she’d be dead within a few years. If Katie finds it so hard to lose weight when so much of her life is controlled, what hope is there for someone with PWS with no control at all? 22-year-old Sachin Vyas has tried and failed to live independently with PWS. He has diabetes and high blood pressure, brought on by obesity. He has piled on 11 stone (154 pounds) in 12 months and now tips the scales at 22 stone (308 pounds). To live with a person with PWS whom we cannot control, it’s basically like living like a slave. At home, you could always coax him, or appease him and deal with him. But when they’re outside, it is beyond your control. Thanks to disability benefits, Sachin gets over £80 a week to spend on what he likes. I like to buy sweets, chocolates, crisps, biscuits, alcohol, sweet drinks… Sachin’s sixth form college are doing what they can to stop him eating himself to an early death. No, you’re not allowed to drink that. You know that. He’s the devil! I have been told to just watch because he’s not supposed to have sugary drinks, is he? And chocolate, of all things, and he loves his chocolate and sweets. I was under the impression that there was control in terms of him maybe not having any money. But then, I was made aware that he did have money. So he was going in and buying as much as he wanted. So you’ve had a baked potato… Sachin was brought up as a vegetarian, but PWS has changed all that. What pasta is it? Chicken? I thought you were a vegetarian. What’s happened? No I’m not a vegetarian, mate. Why don’t you leave your Crunchy Crunch till later, because that’ll give you a snack. Although the school are trying their level best to support him and coach him, I think it’s going to be very difficult, because Sachin’s too good with money. Back home, Sachin’s father Kishor tries to keep food out of reach. Recently this door was pushed in Because it leads to the kitchen. The whole front bit came out as you can see from one side and that’s opened up the whole thing. These big panes of glass have been broken. Because of his size and that, I’m sometimes scared to be on my own in the house because he has attacked me in the past. The whole family have changed their food habits for Sachin’s benefit. We have reduced a lot of things with sugar in the house. Basically, there’s nil at times. Everybody’s food has basically changed. Everyone’s food except Sachin’s, who raids his own private hoard of snacks. Ginger snaps, ice cream wafers, He’d bring loads and loads of food, which is junk food. Cans of soup… I’m not getting junk food, you know. Beer… As he eats, he’s getting nearer to death and that is the reality of this syndrome. Even Sachin is worried about the consequences to his health. I worry about my weight. I want to be normal but I can’t be normal, because it’s a genetic condition. If I eat more and more, I’ll just…die. So desperate is Sachin, that he’s planning to go to India to meet his spiritual healer. Rebecca’s fate is in the hands of the local health authority. She’s going to a hospital appointment, but her dad’s about to make a big mistake… Daddy’s going the long way. Daddy! Why d’you go the long way? Like many with PWS, Rebecca thinks in a rigid way, and she can’t cope with changes to routine. Daddy! Daddy, I’m gonna be late! He can’t turn round now, can he? He should’ve done that! No you made him do that! Mum: Don’t kick me! There’s only one thing on Rebecca’s mind. What’s for dinner tonight, Mummy? I got you a special chicken chow mein. All for me? All for you, special. Is it, Mummy? Yes. But what’s in it? Rat’s poison. No! What’s in it!?!? Chicken! And what? Noodles and vegetables. Chinese. Rebecca’s social skills are affected by her condition, and her relationship with her younger sisters is getting more difficult. Technically, Rebecca is the oldest so she’s the big sister But mentally, I suspect it’s probably Chloe. Chloe just loves winding her up. She can just snap and that’s it – she don’t want no one near her. “Get out of my bedroom. Don’t touch my toys.” So Chloe and Katie tend to play together whereas Rebecca plays on her own. She loves her jigsaws, our Rebecca. We’ve got hundreds in the wardrobe. And she’s not very good at keeping the pieces all in one box. The Hulk is probably her best friend. Katie Williams overcame years of isolation to succeed at college. If only she could control her weight. But not all her dreams are possible. I don’t mind if I have a child disabled, as long as I have a child of my own. The only thing I want in life is children. I always told her, right from a small child, that she wouldn’t be able to have children, because they didn’t want it to come as a shock in adulthood. When I see Katie struggle, it’s heartbreaking. I can’t get up! She has an awful lot of weight to be carrying, and the body just can’t cope with it. Katie really did very well at college, and achieved so much in so many other ways and through her own very hard work, and willingness, and cooperation and so on, but in every other way she could be totally independent, apart from controlling her weight.